Reaching Down the Rabbit Hole E-Book

Allan Ropper

• Herausgeber: Atlantic Books
• Kategorie: Wissenschaft und neue Technologien
• Sprache: Englisch

Seitenzahl: 429

Veröffentlichungsjahr: 2014

Beschreibung

SHORTLISTED FOR THE 2016 BMA MEDICAL BOOK AWARDS One of the world's leading neurologists reveals the extraordinary stories behind some of the brain disorders that he and his staff at the Harvard Medical School endeavour to treat. What is it like to try to heal the body when the mind is under attack? In this gripping and illuminating book, Dr Allan Ropper reveals the extraordinary stories behind some of the life-altering afflictions that he and his staff are confronted with at the Neurology Unit of Harvard's Brigham and Women's Hospital. Like Alice in Wonderland, Dr Ropper inhabits a place where absurdities abound: a sportsman who starts spouting gibberish; an undergraduate who suddenly becomes psychotic; a mother who has to decide whether a life locked inside her own head is worth living. How does one begin to treat such cases, to counsel people whose lives may be changed forever? Dr Ropper answers these questions by taking the reader into a world where lives and minds hang in the balance.

Leseprobe

REACHING DOWN THE RABBIT HOLE

ALSO BY DR. ALLAN H. ROPPER

Principles of Neurology

ALSO BY B. D. BURRELL

The Words We Live By Postcards from the Brain Museum

First published in hardback in the United States of America in 2014 by St. Martin’s Press, LLC.

First published in Great Britain in 2014 by Atlantic Books, an imprint of Atlantic Books Ltd.

Copyright © Dr. Allan H. Ropper and Brian David Burrell, 2014

The moral right of Dr. Allan H. Ropper and Brian David Burrell to be identified as the authors of this work has been asserted by them in accordance with the Copyright, Designs and Patents Act of 1988.

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior permission of both the copyright owner and the above publisher of this book.

1 2 3 4 5 6 7 8 9

A CIP catalogue record for this book is available from the British Library.

Hardback ISBN: 978 1 782 39547 8 Trade Paperback ISBN: 978 1 782 39548 5 EBook ISBN: 978 1 782 39549 2

Printed in Great Britain

Designed by Kathryn Parise Atlantic Books An Imprint of Atlantic Books Ltd Ormond House 26–27 Boswell Street London WC1N 3JZ

www.atlantic-books.co.uk

CONTENTS

Introduction: The QueenWhat neurologists do1.Six Improbable Things Before BreakfastArrivals, departures, and delays on the ward2.Like a Hole in the HeadWhere baseball and neurology converge in a game-saving, over-the-shoulder catch3.The State of ConfusionTwo characters in search of a neurologist4.My Man GodfreyA poor sort of memory that only works backwards5.What Seems to Be the Problem?A politically incorrect guide to malingering, shamming, and hysteria6.Do No HarmA walking time bomb tests the limits of good sense7.A Story Is Worth a Thousand PicturesNine songs of innocence and experience8.EndgameFacing down Lou Gehrig’s disease9.The Examined LifeWhat it takes to survive a motor-neuron death sentence10.The Curse of the WerewolfOn the front lines in the battle against Dr. Parkinson’s disease11.For the Want of a NailA hard-knock lesson on the way to the morgue12.The Eyes Have ItWhen is somebody not dead yet?13.Boats Against the CurrentBased on a true storyAcknowledgments

REACHING DOWN THE RABBIT HOLE

INTRODUCTION:

The Queen

What neurologists do

“Hello, I’m Dr. Allan Ropper. How are you?”

“That’s a grammatical question. Plus and minus.”

“Is your mind clear?”

“I guess so. There are a lot of unexplained issues around me, but . . . I’m communicating appropriately.”

His name is Dr. Vandermeer. He is in his mideighties, and he is a genuine Boston Brahmin. I know his type well. Over the past fifty years he has built a national reputation as a top-drawer researcher and a caring physician, universally admired, all the while taking somewhat heedless care of his own body. He is a man of arts and sciences, but also a man of tastes and habits acquired from his father and grandfather, which is to say, he is a nineteenth-century Yankee living a twentieth-century life in the twenty-first century, and is only vaguely aware that he can no longer manage in the ten-room house he has occupied for the last fifty-two years. When he retired in his seventies, he settled upon a daily routine that failed to anticipate his declining faculties. He is as unwilling to accept this fact as he is to acknowledge the unruliness of his eyebrows or his surplus of nose and ear hair, a clinical indifference that is not unusual in aging doctors.

“Do you know where you are?”

“At Brigham and Women’s Hospital.”

“The date?”

“The date? No, I couldn’t give you that.”

“Year?”

“Again, it’s such a confusing sequence of events that it has confounded me to where my orientation isn’t what it should be.”

“No problem. Are you in any pain?”

“No.”

Doc Vandermeer was brought here over his own objections after his wife found him sitting on the toilet some seven hours after he had ventured from bedroom to bathroom. He had spent the night there.

“Have you had any hallucinations?”

“I don’t think so, but then people generally don’t.”

“Touché. Have you had any convulsions?”

“No.”

“And you know about this meningioma in your right temporal lobe? You have a meningioma there about the size of a lemon. Were you aware of that?”

“I’ve had two growth issues that are pertinent. One is in the pancreas and the other is there.”

“But you seem a little off, cognitively, and our struggle is, could it be the meningioma that’s doing it?”

“As they say, that’s your problem.”

He’s right. It is my problem. I am his neurologist. It is my job to parse his convoluted responses, fit them into the clinical picture, prioritize his issues, and come up with a plan that lets him live the life that he wants to live, to the extent that is possible. In his peculiar way, he has indicated that he is oriented to his location, but not oriented to the time or to his situation. His highly formalized locution, entirely characteristic of his tribe, may seem quaint, but it is exaggerated just enough to suggest that he is compensating for a language deficit that he is aware of but seems indifferent to. He knows that he has a benign tumor in his pancreas that poses less of a threat than the meningioma in his brain. The brain tumor will not kill him anytime soon, but it will continue to diminish his powers of thought.

“We’ll find out what’s what and let you know,” I say. “Great to meet you.”

It will take us some time to sort it all out, but this is a start. Hannah, my senior resident, gives me a nod, signaling that it’s time to move on to the next patient.

“Hello, there. I’m Dr. Ropper. I’m one of the neurologists. And you’ve met Hannah Ross, our chief resident. Do you mind if we visit with you?”

His name is Gary, and he is one unhappy customer. He is thirty-two years old and has spent more time in hospitals than he cares to recall.

“How are you doing?” I ask.

“I can function,” Gary replies listlessly, “but not to design specifications.”

“Interesting phrase. Where did you get that from?”

“Gene Roddenberry.”

It fits. Gary is a computer programmer who has so immersed himself in Star Trek that he knows the Starship Design Specifications by heart. He has the body of a Buddha, the eyes of a koala, and an air of resignation that is probably his baseline. He might as well be wearing a T-shirt emblazoned with the motto: IT IS WHAT IT IS. He has had epilepsy since childhood, culminating in the surgical removal of part of his brain when he was seventeen years old. A U-shaped scar stands out prominently on the left side of his close-cropped scalp. He has enough experience with his condition to know when he needs to go to the hospital, although he would rather not go. When he does, he most definitely does not want to be admitted, but Hannah has admitted him anyway, and there is a lingering animus on his part.

“I understand you had several events yesterday,” Hannah says. “A lot of seizures?”

“Well, not many for me. What’s considered many?”

“It all depends on how many is many for you. I was under the impression that you did have many for you . . . in a row. Is that not true?”

“No. Even when I have clusters it’s probably four or five.”

“Is that what you had yesterday? Four or five?”

“Well, that’s not what brought me to the hospital, if that’s what you’re getting at.”

Gary is in a mood, so I’m happy to let Hannah carry the ball. This is her ward, these are her patients, I am the attending physician, and while I took the lead with Dr. Vandermeer in deference to his stature, I now take up a position at the foot of the bed and watch.

“That is what I’m getting at,” Hannah continues in a sing-song tone as if trying to engage a curious child. “So what brought you to the hospital today?”

A pause. Gary seems bothered less by Hannah’s tone than by her question. “It was late in the afternoon,” he replies with mock sarcasm. “I had a really bad headache and I was feeling very tired. I was clumsy and I couldn’t stand up straight and my hands were vibrating.”

“And that’s not what usually happens with your seizures?”

“Well, I don’t get so much warning with the seizures, although some days I can tell if I’m walking up to the edge of a seizure, but it doesn’t always lead to a seizure. Sometimes it leads to a lot of seizures, sometimes nothing.”

“How many seizures do you usually have in a given day?”

“There is no usual. I can go as long as five days without any seizures at all.”

Hannah’s directed line of inquiry seems to have snared his attention. He is clearly not used to having anyone take such a keen interest in his troubles.

“And what’s your usual when you do have them?” she asks.

“My record is eighty-four seizures in a day.”

“Is that typical?”

“There is no rhyme or reason to it. Some days I have none, some days I have a lot.”

“But say in the course of a month.”

“You keep wanting me to do that. You keep wanting me to put some pattern to it. Believe me, I wish I could put a pattern to it. Would you like to see my seizure log?”

“That won’t be necessary.”

I’m staying out of it. Hannah is firm yet restrained and unflustered; Gary is exasperated to the point of annoyance, almost anger, without the emotional edge to attain real anger. He acts besieged, yet lacks the reserves of panic that fuel true paranoia. It’s as though he has programmed himself to be confrontational, but the confrontational part of his brain is so compromised that it can’t run the code.

Hannah presses on. “What I really want to understand is whether you ever have a month without seizures.”

“A month? No. I’m lucky if I go a week.”

“And then when they come you can have one or you can have a ton?”

“Yes.”

“Any missed doses of your meds?”

“Everyone wants to blame the epileptic. ‘You wouldn’t have seizures if you didn’t miss your doses.’ Is that it?”

She doesn’t bite. “No. You’d probably have seizures anyway.”

“That’s true. I don’t miss doses, and I have seizures.”

We have the picture now. Fortunately, Gary does not. If he did, he would know that Hannah suspects, as do I, that some of his seizures are fake. It is time for us to get going again.

“It was really great to meet you,” I say. “I wish the rest of our patients had your outlook.”

“You mean felt that there’s nothing you guys can do?”

“No, I mean had your level of self-awareness.”

Gary has what was once called a temporal-lobe seizure personality. He is intelligent, mildly paranoid, has good word usage, and most of all is extremely literal. He is wrong that there’s nothing we can do, although this has long been a knock on neurology: that we can usually tell what’s going on in someone’s brain, but that we can’t do much about it. I won’t bother to disabuse him of the idea because it would take a book to do so. This book, for example.

Gary is right about one thing. His mind is not working to design specifications, although in that regard he is hardly unique. There are twenty-nine other people on the ward right now who could say the same thing.

They call this place the Brigham, short for Brigham and Women’s Hospital, an amalgam of the names of a few historic hospitals that merged decades ago—the Peter Bent Brigham, the Robert Breck Brigham, and the Boston Hospital for Women—and it spans the length of a city block that abuts the Harvard Medical School campus. This massive enclave of teaching hospitals, which also includes Beth Israel Deaconess Hospital, the Dana Farber Cancer Institute, Boston Children’s Hospital, and the Joslin Diabetes Center, serves as a training ground for Harvard’s medical students. It is a city within a city, or at least a city on the very edge of a city: a district of glass, stone, and steel towers that occupies the eastern bank of a lazy, muddy waterway that separates Boston from the suburb of Brookline. At clinics and community hospitals throughout New England, whenever a doctor looks over a patient chart, sighs, and says, “Send it to Boston,” there is a good chance that the patient will end up here.

On any weekday morning the inbound traffic is daunting, like a rising tide in a teeming estuary, bearing with it an influx of patients, visitors, and family members, not to mention staff, that will be carried away with the ebb at day’s end, leaving behind isolated pools and puddles of low-level activity.

This is one of those pools—the neurology inpatient ward. It occupies the tenth floor of the hospital tower. Built in the 1980s, the tower has a cross section in the shape of a four-leafed clover, each leaf being a pod, each pod consisting of a dozen fanned-out rooms, each room visible and easily accessible from a semicircular nurses’ station, a novel idea that now seems somewhat dated. The patients here, like Gary and Doc Vandermeer, are in tough shape, and they spend much of their morning waiting for our visit, and the rest of the day wishing they were someplace else, even though this is the best place they could be. It is a place where the strangest and most challenging cases are sent to be sorted out, cases whose complexity would tax the resources of smaller hospitals.

I am a clinical neurologist and a professor of neurology. Most people have little idea what that means, but among other things it means that I am an authority on what the brain does right and does wrong: language, sensation, and emotion; walking, falling, weakness, tremors, and coordination; memory, mental incapacity, delays in development; anxiety, pain, stress, even death. The practice of my craft, the clinical part of it, is the systematic, logical, deductive method that was in the past applicable to all branches of medicine, but now resides mainly in neurology. The paradoxical part of it, the unique challenge, is that my primary sources of information—my patients’ brains—are quite often altered, sometimes bizarrely, as a result of disease. This creates an incredible and self-referential conundrum. How do you begin to understand a sick brain? The only viable answer, as this book will show, is that you do it by engaging the person inside, and you do it on a case-by-case basis.

“He hasn’t felt emotion for at least a month.”

This is the girlfriend speaking. She is in her midthirties, as is her boyfriend, the man without emotion. They are not well-to-do, but they get by. She speaks with a strong North Shore accent, and despite her downscale style of dress, she has above-average verbal skills: she listens carefully, processes information quickly, and answers helpfully. They have lived together for five years. They have a child. They are resigned to their fate.

“What do you mean by that?” I ask her.

“No sadness over what’s happening. No anger anymore. When he was first diagnosed he had a lot of anger issues. But recently he’s just been happy, not out of control.”

“Happy, or just nothing?”

“I would say content,” she replies. “He’s easygoing. The TV used to make him laugh. I saw happiness then. Not now.”

“When he expresses an emotion . . . ?”

“He may smile, nothing else.”

“In speaking to you, speaking to each other?”

“No. He really hasn’t initiated a conversation in the last month.”

“That’s typical for a butterfly glioma infiltrating the frontal lobes,” I say, referring to his spreading, malignant brain tumor. “So we’re really seeing him in good shape now. Is that true?”

“Yeah, he’s better right now, on steroids. This is way better than he’s been in a long time now.”

I turn to him. “Can you tell us a joke, Dennis?”

“Hopefully not,” he replies with little affect. Dennis seems indifferent even when he grins. His head is shaved, and his cranial stubble reveals the dark shadow of a receding hairline. He has a tattoo on his left forearm, in Gothic text, that reads: SICK.

“There’s enough rattling around in there so that your thinking seems really good.”

“Yeah.”

“What’s missing? What’s not right in your head?”

Dennis pauses, inhales deeply through his nostrils, thinks about it, and lets it out slowly, but says nothing.

“You have trouble expressing yourself in language, or expressing yourself in emotion?”

He takes his time. “I think it’s in emotion.”

To the girlfriend again: “I understood from the residents that there’s a conversation going on about hospice. How did you get to that? Was he becoming unmanageable at home?”

“Over the last few weeks, yes, he was.”

“On account of what?”

“Behavior. He wouldn’t listen to me. He kept getting up and opening the fridge, wanting to take his medicine. Then just being a little bit agitated. But as far as stopping treatment, we talked to the oncologist last night. The chemo affected him very badly. The doctor said that there aren’t any drugs left that won’t make his life really terrible. So we’re talking about quality of life at this point, because the tumor is so large, and it’s inoperable.”

I turn to Dennis: “Are you engaged in these conversations?”

“Not that one, but we did talk about treatment a bunch yesterday with Dr. Nadgir.”

“Before,” she says, “he wasn’t on a high dose of steroids, he wasn’t clear at all. But now, on the steroids, this is a complete turnaround.”

“Are your plans staying the same?” I ask her.

“I’m thinking maybe we can bring him home and have hospice come in.”

“Can you behave yourself if you go home?” I say to Dennis.

He grins and shakes his head in a “no” gesture.

“No? So this is some combination of the tumor and his character. I mean, you are a character, Dennis, right?”

His grin springs from memory more than feeling. He knows that it’s only a matter of months. The irony is that the location of the tumor has neutralized the part of his brain that would allow him to care. So he was a character, but not so much anymore.

What Dennis, his girlfriend, and everybody else on the ward need, more than anything else, is to tell us their stories. Many of them have driven for an hour, two, even three hours to the Hub of the Universe (as Boston styles itself), and they want to be heard. What they hope, what they expect, what they deserve, is that we take the time to listen, because the act of listening is therapeutic in itself. When we do it right, we learn details that make us better doctors for the next patient. The residents may not get this yet. They are focused on diagnosis and treatment, on technology, on scales, titers, doses, ratios, elevations, and deficiencies. All well and good, I tell them, but don’t forget to listen.

Hannah and the team are huddled around a computer monitor in a cubbyhole near the nurses’ station on the tenth floor. They have made it through medical school and earned their MDs, chosen to go into neurology, and this is now finishing school for them, a prestigious appointment at a top-tier hospital. My role is to look over their shoulders, provide them with someone to model, give them a hard time (or, pedagogically speaking, challenge their assumptions).

As they play at the computer, a patient sits twenty feet away, just beyond a glass door and curtains. He was admitted three hours ago with the sudden onset of a speech difficulty and a complete personality change. The team hasn’t visited him yet. Instead, they are transfixed by the images of his brain up on the screen, like passengers glued to the in-flight movie as they fly over the Grand Canyon at sunrise. It reminds me of an old joke: Two grandmothers meet on the boardwalk, one pushing a baby carriage. “That’s a beautiful granddaughter,” the friend says, and the other replies, “This is nothing, you should see her pictures.”

“Anything?” I ask.

“I don’t think it’s a stroke,” Hannah says. “Maybe there’s a low-grade glioma in the right frontal lobe, a little bit of hypodensity.”

“Would you put a dime on anything now?” I ask her.

“Tumor.”

“What do you think?” I ask Rakesh, a second-year resident.

“I think it could be a combination of tumor and postictal changes,” he replies. “How about you?”

“Me?” I say. “I’ll be glad to hold the money for you.” What I want is for them to step away from the monitor and into the room, sit by the bed, talk to the patient, and examine the man instead of his pixels.

“Here’s a small side bet,” I add. “I don’t think the enhanced MRI is going to help us. Look, you’ve got the whole package here—the scans and the tests—and you’re still not sure what’s going on. That’s why neurology is the queen of the medical specialties.”

“Not the king?”

“No, it’s the queen because the queen is elegant, and it is the last place in medicine where your personal synthetic intellectual effort is value added, and despite all these gee-whiz gizmos, there really are no tests. You’re going to have to sort this one out at the bedside.”

Hannah has heard this speech before, and has a pretty good idea what it means. It means that if a patient comes in and can’t speak clearly or zones out mentally or can’t feel emotion, there are no standard tests. The symptom has to be reframed in terms of brain function. The only way to accomplish that reframing is through the elegant choreography of the neurological exam, not with scans, but through the painstaking examination of the patient. Every gesture, every movement, every inflection of speech, every reflex, all of these point to the precise location of the problem in the nervous system, and to its cause. The physician’s art is to synthesize the symptoms and signs in a larger framework of the patient and the structure of the nervous system. The tests are merely confirmatory, all the way from carpal tunnel syndrome to brain death.

But for now, the team is still learning about the brain, if not the entire nervous system, observing up close the ways it can malfunction. In time they will figure out how to interact with the person inside the brain, with the brain inside the person. They will find their comfort level with the families—how to give good news, how to give bad news. Someday, they will be the ones who have to tell a patient that nothing will ever be the same.

“You have a brain tumor.”

“You have motor neuron disease.”

“You have Parkinson’s.”

“You’ve just had an irreversible stroke.”

What does it mean to be a doctor to these patients? More importantly, what does it mean to be the patient faced with these seismic problems, and how is a connection made with the physician who embodies the knowledge that can make it better?

This book is about the process and progress of my craft. Like clinical neurology itself, it proceeds on a case-by-case basis. The stories are real, the dialogue mostly verbatim, and although the identities of almost all the patients are disguised, the challenges and issues facing the neurologist in a teaching hospital are on full display.

Where does it begin? It begins with a bowling alley manager who gets confused, with a right fielder who starts spouting gibberish, with an undergraduate who suddenly becomes psychotic, with a salesman who drives around and around a traffic rotary, unable to get off, with a college quarterback who can’t stop calling the same play, with a psychiatric social worker who notices a tremor in her pinkie finger, with an ex-athlete who cannot grasp the pull tabs on his newborn daughter’s diaper, with an Irishman who slips on the ice and cracks his head. They wind up here, on the inpatient ward or in the neuro-intensive care unit, passing through in a parade that never ceases to challenge, astound, and enlighten me.

Where does it end? It doesn’t.

Dr. Vandermeer, one of the last survivors of the generation that trained me, sits up on 10 West with a brain tumor the size of a lemon. Gary the programmer is having electrical leads affixed to his head on 10 East. On 10 North, Dennis of the butterfly glioma, along with his girlfriend, have decided to put off hospice for a few more weeks, and they are preparing to go home. Speed rounds have ended, and I am in the elevator, bearing coffee, on my way to 10 South, where Hannah is waiting, patient list in hand. When she sees me, she grabs the hospital ID on the lanyard around her neck, sweeps it over a wall-mounted detector, and the doors to the neuro-ward, like all hospital doors, swing open in counterpoint. As we walk through, she exchanges the patient list for the coffee cup I hold out to her, and I ask the question I have asked countless times:

“So what have we got?”

1

Six Improbable Things Before Breakfast

Arrivals, departures, and delays on the ward

On the third of July, a day after a routine colonoscopy, Vincent Talma was playing right field in a company softball game. A short, intense man with thick gray hair and a perpetual scowl, he did not look as though he was having fun, or even capable of having any fun. Whenever a teammate said something amusing or cracked a joke, Vincent would laugh without smiling, as if to say, “funny, funny, ha, ha.” When he disagreed with a call by the umpire, he would throw up his arms in disbelief, kick the dirt, and swear under his breath, not for show or for the approbation of his teammates, but out of real anger and disgust. No one called him Vinnie, few called him Vince, and when he stood at the plate, none of his teammates dared to cheer him on by name.

As the game wound into the late innings, Vincent’s behavior began to change, subtly at first, then dramatically. By the time he was dropped off at his house, his wife was startled to see a bemused look on his face, an air of innocence in place of his usual gruffness. He gave one-word answers to her questions, avoided eye contact, and seemed quite unlike himself. He was smiling too much.

“Are you okay?” she asked.

“Sure, fine,” he replied.

“Did you win?”

“Fine, yes.”

“Did something happen?”

“Fine.”

The more she persisted, the more Vincent perseverated.

“Did anything happen at the game?”

“Fine, yeah fine,” he mumbled with a sheepish smile.

She called their primary care physician, who told her to get him to the emergency room immediately.

“Vincent, we need to go,” she said.

“Fine. Okay.” Still smiling.

At East Shore Hospital an MRI showed an ambiguous blotch on the left frontal lobe of Vincent’s brain, and at the suggestion of one of his sons, a pediatrician, the family requested a transfer to us. He arrived sometime around 10:00 that morning and was brought up to the ward.

A week earlier, Cindy Song, a sophomore at Boston College, had started acting a bit withdrawn. Her roommate was concerned enough to call Cindy’s sister. The first phone call was not too worrisome. “Not a big deal,” the sister said. “She gets that way. Just give her time. She’ll be okay.” The next call could not be taken so lightly.

By morning, Cindy wouldn’t leave her room, and would not or could not tell her roommate why. Alternately anxious and distracted, uncharacteristically morose and sullen, she spent the day in bed. That evening she refused to eat, and her roommate made the second call, this one to Cindy’s mother, a first-generation Korean immigrant. Despite the language difficulty, there was no mistaking the concern in the roommate’s voice. Cindy’s mother took the next commuter train from Framingham, exited at Yawkey Station, took the Green Line out to Chestnut Hill, walked up the steep hill from the terminal, past the Gothic spire of Gasson Hall, and down the long, winding road to the dorms. When she got to Cindy’s room and sat down in front of her daughter, all she got back was a blank stare focused on the wall behind her. Her daughter’s eyes were wide open and her pupils dilated. She was shivering mildly and sweating all over. Finally, she spoke.

“Mom, they’ve been after me for weeks, creeping in through the cinderblocks, taking my clothes off.”

“What are you talking about, honey?”

“My clothes, my clothes,” she said desperately, “can’t you see them?”

Like all universities, Boston College has a health center that provides minimal services overnight, on holidays, on weekends, and during the summer, relying on referrals to local emergency rooms for anything serious. The after-hours nurse, who was used to such things, assumed that Cindy had been using recreational drugs and was “just flipping out.” Nothing unusual as far as the nurse was concerned, but Cindy’s mother was outraged. Convinced simply from cultural experience that there were no drugs involved, she would not let that stand. Cindy was so jittery and sweaty that the nurse gave in and called an ambulance to take her to the Brookline Hospital emergency room. Once there, Cindy remained agitated, stopped responding to questions, and started thrashing, as though reacting to hallucinated visions. This prompted a round of phone calls to the eight local psychiatric hospitals to see if there was a bed for an acutely psychotic young woman. Such beds are hard to come by, and it took a hard sell by the emergency room doctor to secure the promise of one by the next afternoon, “if you could just hang onto her and give her Haldol in the meantime.”

As daylight broke, Cindy was close to berserk. Her arms had to be restrained with straps, and she was soaking up tranquilizers like a sponge. Finally, the ambulance came to bring her to the psychiatric hospital. After a short interlude of relative calm, the psych nurses became alarmed when Cindy’s jitteriness escalated into full-blown myoclonus—arms and legs flinging up off the bed, her head jerking back violently. Her pupils were huge. If it had been a drug overdose, they realized, this would have abated by now. Instead, the hallucinations continued, and Cindy was excessively restless and sweaty. I got a call at about 9:30 a.m.

“Is she salivating like she has rabies?” I asked the psychiatry resident.

“Yes, like a dog,” was the reply.

“You’d better send her over.”

By the time Vincent Talma and Cindy Song had settled in at the Brigham, Arwen Cleary had been there for four days. She came by ambulance on the morning of July 1, and was admitted to neurological intensive care from the Emergency Department later that evening. Of the three cases, hers was the least clear-cut, the most troubling, and one that had the potential to become an absolute shambles. According to her medical records, her problems had begun two years earlier, when she showed up at a central Massachusetts hospital with disabling nausea, difficulty walking, and vomiting.

Arwen Cleary had been a professional figure skater as a teenager, had retired from the Ice Capades upon its dissolution in 1995, had then raised three children, gotten divorced, and moved with her two younger children to a ranch house in Leominster, a distant suburb, where she worked part-time at a local health club. Her medical history was unremarkable: once a smoker, she had quit ten years earlier. Her travels had taken her no place more exotic than Bermuda and no more distant than Orlando. Her only hospitalizations to that point had been in maternity wards. She was remarkably fit and in seemingly good cardiovascular health, if judged only by her appearance and vital signs. But shortly after a visit to a chiropractor, she had suffered a vertebral artery dissection, a form of stroke.

Chiropractic neck adjustments are not a common cause of stroke (maybe one in every twenty thousand treatments produces one), but the high rotary force involved, one with just the right vector and amplitude, can strip off the inner layer of a blood vessel, causing it to tear and collapse into the channel, impeding the flow of arterial blood to the brain. At her local hospital, Ms. Cleary was started on a blood thinner, and after a long inpatient and rehab stay, she recovered her motor skills and balance, and was sent home.

All went well for two years, until she returned to the hospital with sudden right facial drooping and difficulty finding words, sure signs of another stroke, but this time a stroke of a very different kind. A portion of one of the language centers of her brain had been deprived of its blood supply. Her speech was now noticeably impaired. Within a few days, she showed signs of improvement, and was again discharged on a blood thinner.

Ten weeks later, to her infinite frustration, it happened yet again, and she arrived at the same hospital in the middle of the night with another language problem, this time even more pronounced, as well as right arm weakness. The scans now showed that several other blood vessels had been stopped up, causing a scattering of new strokes. At that point her doctors became even more worried. Why would this be happening in someone so young? But they could locate neither a cause nor a source. They subjected her to exhaustive tests, the usual suspects for stroke were rounded up, an echocardiogram was ordered, and she was given a portable heart monitor. Everything came back normal. It was decided that the previous chiropractic stroke (the dissection) was unrelated to her current problem. Among the staff, the consensus was: “We’re going to need a bigger boat.” So they sent her to us.

There is an old joke among stand-up comics that goes: “Dying is easy, comedy is hard.” If we were as inner-directed as comedians, we neurologists might say, “Trauma is easy, neurology is hard.” Every one of our patients has, in effect, fallen into a hole, and it’s our job is to get them out again.

In Alice’s Adventures in Wonderland, Alice jumps into a rabbit hole and finds herself in a bizarre realm in which nothing is what it seems, where everything bears little relation to the outside world. It is a place where, as the Red Queen mentions to Alice, it helps to believe six impossible things before breakfast. Unlike the Queen, I have no need to believe six impossible things before breakfast because I know that, on any given day, I will be confronted by at least six improbable things before lunch: a smiling man whose speech difficulties seemed to have been brought on by a colonoscopy, a thrashing young woman whose psychosis seemed to come out of nowhere, a figure skater with a slow-fuse time bomb in her body that was knocking off her faculties one by one. The first of these, I should note, was indeed impossible, and I didn’t believe it for a second, but the next two were quite possible, and by the end of the morning, I would encounter at least three more improbabilities: a woman who could only be cured by a hole in the head, a case of amnesia brought on by sex, and a man who was adamant that I was two very different doctors.

We treat people with seemingly implausible ailments all of the time. Each day they show up in a predictable parade of signs, symptoms, and diseases: an embolus, a glioma, a hydrocephalus; a bleed, a seizure, a hemiplegia. That’s how the residents refer to the cases, as in: “Let’s go see the basilar thrombosis on 10 East.” When viewed in terms of actual patients, however, no day is quite like any other. After the bedside visit, the thrombosis suddenly has a name, the glioma has a wife and children, the hydrocephalus writes a column for a well-known business journal. Our coed suffering from psychosis turned out to be a Rhodes Scholarship candidate, the case of multiple strokes became a charming woman who had competed in the Junior Olympics, and the man for whom a smile was a troubling symptom owned a personal empire of six Verizon wireless stores.

“Good morning, Mr. Talma,” Hannah said, “do you remember me?”

“Yes, good, good, fine,” Vincent replied. He was sitting up in bed, watching television with a smile of bemused innocence. Vincent Talma was a picture of contentment. His room on the tenth floor of the hospital tower commanded an outstanding view of Fort Hill Park in Boston’s Roxbury section, but Vincent took no notice. Along with twenty-nine of our other patients, he had been waiting for a visit from the neurology team on their morning speed rounds.

Hannah was in charge. Her service, the culmination of three years as a neurological resident, had started a week before I came on board. A “service” involves running the neurology inpatient ward, admitting and discharging the patients, and directing a team consisting of three junior residents, two medical students, and a physician’s assistant—a cohort that could barely squeeze into Vincent’s curtained-off half of the room.

My colleagues and I had some doubts about Hannah when she first came to the program three years earlier. The most superficial of these doubts focused on her style of dress. In a profession where sartorial flair is an unexpected and somewhat suspect concept, Hannah’s clogs, leggings, and wraps seemed needlessly exotic, and sowed uneasiness among the Dockers, Skechers, and scrubs crowd. Perhaps even more alienating was the fact that Hannah did not drive a car, and instead rode her bike from her apartment in Boston’s North End to the Brigham, usually well before the sun rose or long after it had set, in any kind of weather short of a blizzard. Such stoicism flew in the face of the unhealthy lifestyle adopted by most of the residents and teaching faculty, who tend to favor pastries over granola, Coke over water, and elevators over stairs.

I could see that over the course of the previous week, Hannah had begun the transition from resident to full-fledged physician. I could see it in her bearing, in the assertive physicality with which she carried out her examinations, in the firmness of her tone with some of the more difficult patients, and in the controlled sympathy she adopted in family meetings when she had to deliver bad news. She had turned out to be one of our strongest clinicians.

Although she hails from the Midwest, Hannah Ross has a northern European flair, somewhat Dutch, in that she is tall, lithe, wears fashionably businesslike glasses, and seems indifferent to the possibility that anyone might appreciate the effort she has made in choosing her look, probably because the effort is now merely a habit. She moves swiftly from room to room, from pod to pod, from the nurses’ station to the rolling laptop cart, where she displays an instantaneous command of electronic medical records, and can bring up an MRI scan and zoom in on a tumor or a cerebral hemorrhage with no wasted effort.

“What are you watching?” Hannah asked Vincent, in an inflection she would later inform me was Kansan rather than Missourian.

“The Bunkers.”

“Do you mean All in the Family?”

“Yes, yes, . . . the Bunk . . . Yes.”

Vincent’s form of speech difficulty, known as Wernicke’s aphasia, sounds like gibberish, but not pure nonsense. It can include halting phrases that almost make sense, echolalia (repeating someone else’s just-used words), perseveration (giving the same answer to a succession of different questions), and play association (cracking wise). While he knew the answers to many of our questions, most of his responses didn’t come out quite right, yet he seemed unaware and unconcerned.

“What’s your name?” Hannah said.

“Vincent.”

“Good. Where are we? What place is this?”

“Vincent . . . uh, yeah . . . Vince.”

“What day is it?”

“Avince . . . Vince.”

“Okay. Look at my hand. Now follow my thumb.”

“Gee, you’re so dumb.”

Gilbert, the medical student who had made the initial exam, recorded this as “orientation times one.”

“To one what?” I later asked him.

“To himself,” he said.

“Have you ever met a patient who wasn’t?”

“I don’t think so.”

“No, you haven’t. It doesn’t exist.”

The phrase A and O times three means “awake, oriented to self, oriented to place, and oriented to time.” Some people add a fourth: oriented to situation. The problem is that everybody is “oriented times one” unless they are hysterical or dead.

Vincent knew who he was. He was sharp enough to find himself amusing. Did his colonoscopy earlier in the week bring this on, or, more to the point, did the anesthesia bring it on? My guess is that it was just a coincidence. A straw poll of the team leaned toward a diagnosis of tumor, possibly stroke, maybe a seizure, but they were basing their guesses on Vincent’s MRI. I had seen the scans and knew they did not hold the answer. On the other hand, Vincent’s wife, who was sitting in an armchair at the foot of his bed, did.

“He had a bad headache from the beginning,” she told me, “and a fever.” The residents had neglected to mention this, but it was important.

“How about a virus?” I suggested. “I think this is probably an infection.” Herpes encephalitis was my hunch. It would connect the headache and low-grade fever, neither of which fit with a tumor or a stroke. “Ignore the scan for now,” I told Hannah. “When there’s nothing obvious there, it can be a distraction. Stick with the patient’s story and the bedside exam.”

We started him on acyclovir, an antiviral medication, and he soon improved. Five days later, Vince was discharged, talking normally again, and, for better or worse, just like his old self.

“I just ran into your Mr. Talma in the elevator lobby.” Elliott, a colleague who seems to keep closer tabs on my patients than I do, had buttonholed me in the corridor outside of the ward. “When I gave him a shout-out,” he said, “you’d think I’d asked him to put up bail for the Unabomber. The guy comes in here a pussycat, and when you finish with him he’s Mr. What’s-It-To-You-Pal. No more smiles, no more jokes. What did you do to him?”

“We cured him,” I said. “Apparently, that’s his baseline. I told his wife that if he started being nice to her again she should bring him back in immediately.”

I was out on the ward at about 9:30 that morning when the call about Cindy Song arrived from the other hospital.

“Is she salivating like she has rabies?” That was my first question, and would turn out to be my only one.

“Yes, like a dog,” was the reply.

“Holy cow!” I said. “It’s an ovarian teratoma. You’d better send her over.” It was a snap diagnosis, possibly wrong, but there was no harm in raising on a pair of aces. I had a pretty good idea what the other cards would be: memory deficits, gooseflesh, a high heart rate, and no family history of psychosis. The drooling alone was a tip-off.

A teratoma is an unusual tumor that contains cells from the brain, teeth, hair, skin, and bone. Most teratomas are harmless, but they have the potential to wreak havoc by causing encephalitis. When you see it, the syndrome is unmistakable: an ovarian teratoma stimulates an antibody that will produce the very ensemble of symptoms that were described to me over the phone.

Two hours later, when she was wheeled into the ICU, Cindy looked toxically ill, with a heart rate of 135 beats per minute and blood pressure of 160/90. She was sweating, salivating, and shivering wildly. Her eyes were wide open but she was by now entirely unresponsive. Her jittery limbs seemed as if they wanted to convulse. Joelle, the senior ICU resident, Hannah’s counterpart down on the ninth floor, immediately intubated her.

The toxicology screen from the other hospital was negative, so I called the gynecology service to get an emergency ultrasound of Cindy’s pelvis. They thought I was crazy. Moreover, I insisted that they do it transvaginally in order to get a good look at the ovaries. An ovarian teratoma can produce memory loss, seizures, and confusion—what neurologists call “limbic encephalitis,” or sometimes the “Ophelia syndrome” (not for Hamlet’s beloved, but for the daughter of the neurologist who described a similar condition). The psychotic symptoms are due to autoimmune antibodies that attach to a receptor in the brain, where they simulate the effects of PCP (aka “angel dust” or “wet”). When that receptor became blocked in Cindy’s brain—when the antibodies hit their target—all of her symptoms became manifest. She went nuts.

“Remove her ovary?” the gynecologist said.

“Right. Do you see that cyst on the ultrasound? It’s not so benign.”

I had to insist that there was now no doubt about it: the ovary-brain connection. First—“Who would have thought?” Then—“What do you know? It’s a real thing.” Eventually, both the resident and the attending gynecologist were convinced, and they were comfortable knowing that Cindy could still have children with her remaining ovary.

This was a rare, rare thing. No one fully understands it, but I know it clinically when I see it, or even hear it over the phone, because I collect arcana. If the problem is properly framed, there are very few other things it could be. It took a bit of cajoling, but in the end, they removed her ovary. The sweating, the salivating, and the wild swings in blood pressure were gone within hours. Her psychosis resolved within days.

Back on ten, Arwen Cleary, our ice skater with the multiple strokes, had gone deeper into the rabbit hole than anyone else on the ward, and I wasn’t confident that we could pull her out of it. According to the notes in her chart, she had by now had three separate strokes, clearly visible on MRI scans, in addition to the vertebral dissection from her neck manipulation. An angiogram had been interpreted as showing vasculitis, an inflammation of the blood vessels. She had a subplural lesion in her left lung, according to the pulmonary specialist. She had a low platelet count, according to the hematologist. “The patient uses humor to cope with her situation,” according to the social worker, and on and on for thirty pages of cut-and-pasted notes from more than two dozen doctors who had examined her over the past two months: too many specialists weighing in with too many disconnected analyses, not adding up to a complete picture. Most of her file consisted of blind alleys and misinterpretations.

She was in rough shape, virtually blind in her right field of vision, and now aphasic. What worried me was that she didn’t have any reserve left, and any little chip-shot stroke was going to be a disaster. The next one, I was convinced, could wipe her out.

“I don’t see any vasculitis here,” I told Hannah. The low platelet count, which if anything would tend to protect against clotting and stroke, was another red herring. “I think the thing to do is just start from scratch. Something is missing. We’ve got a new team, so just make like she’s being seen for the first time, make believe she hasn’t been worked up, fill in all the holes. I think there’s a single origin of these multiple emboli. That’s what it sounds like, that’s what it looks like. There’s something upstream that’s flicking off debris into the blood vessels of the brain, and we just haven’t found it yet. If you told me she had a myxoma, I wouldn’t be surprised.”

Something had to be giving off small flecks that lodged on the walls of blood vessels, effectively narrowing them. That was what had caused the strokes, and that was what had been misinterpreted as vasculitis. It was happening now, and would continue to happen, and the most logical source for the flecks had to be a thrombus (a clot of some kind), a tumor (a myxoma or fibroelastoma), or a bacterial growth due to an infection, probably in or near one of the valves of her heart. Yet my residents insisted that there was nothing wrong on the echocardiogram. After sifting through the case file, we finally got around to visiting her.

“Hello. This is the neurology team. How are you?”

“Not so hot.”

Unlike most of our patients, Arwen Cleary did not look sick. Not only did she look physically fit, but physically vibrant. Rather than sagging into the hospital bed, she balanced on it like a coiled spring, ready to jump out of it if necessary. At the same time she was shy, somewhat abashed at being here. She had had no visitors for over a day, possibly because she did not want her children to see her like this, or, more accurately, to hear her like this, for although she could talk, she could only do so with halting fluency, mostly in monosyllables. She struggled and usually failed to come up with the longer words that best expressed her thoughts.

“I know, it’s tough, not being able to express yourself easily.”

“Oh, yeah. I’m off . . . Oh, my gosh!”

“The dissections . . . I read in the chart that that happened after you got chiropractic treatment. Is that true?

“Right. Yes.”

“How much time elapsed, between the two.”

“It was . . . just a few days.”

“You know your spirits have been marvelous despite all this. How are you doing it?”

“I . . . I . . .”

“You stay optimistic.”

“You have to.” She spoke with an unnatural monotone, somewhat like a deaf person, without accenting any of her words. That was the aphasia. She struggled with all but the simplest responses, and settled for tropes.

“Do you find yourself getting down sometimes?” I asked.

“Some . . . times.”

“Are you depressed?”

“No, not depressed . . . just sick of all this.”

“Discouraged?”

“Yeah.”

“Well, thanks for letting us spend some time with you. We’re racking our brains to figure out what’s going on.”

Stroke offers the most precise and restricted indicator of damage to the brain that nature produces, and therefore allows an understanding of brain function like no other disease. It is highly “readable,” and reading strokes reveals a tremendous amount about the nervous system. One of my professors used to say that the residents learn neurology stroke by stroke. But it is not a simple thing.

Six thousand people have a stroke in the United States every day. The numbers are overwhelming. The country has a stroke belt which runs from North Carolina right through Oklahoma. There are genetic factors and dietary ones. Scandinavians have the fewest strokes, the Japanese have the most. There are at least three broad categories of stroke: one involving blocked blood vessels, another involving bleeding into the brain, and a third—an aneurysm—involving a ruptured bulge in a blood vessel. Although these are all called strokes, they are as different from each other as hepatitis is from gall bladder disease (both of which give you jaundice). And their treatments are entirely different.